Primary ovarian leiomyosarcoma in a woman with uterovaginal prolapse

Abstract

Primary ovarian leiomyosarcoma is a very uncommon and aggressive neoplasm. We presented a right-sided ovarian leiomyosarcoma in a woman in her late 40s. No case has been described in the literature till now of primary ovarian leiomyosarcoma in a woman with uterovaginal prolapse. A total abdominal hysterectomy with bilateral adnexectomy, metastasectomy, excision of large tumour deposit over small intestine followed by resection with ileo-ileal anastomosis and omentectomy was performed. The diagnosis was made based on morphology along with immunohistochemistry. The patient was given adjuvant chemotherapy during postoperative period. Due to rarity, there is a dearth of information on the clinical behaviour and best treatment options for these tumours. This case report highlighted the importance of clinical awareness and aimed to provide a baseline to guide clinical practice as well as future research.

Background

Smooth muscle tumours account for <1% of all ovarian tumours.¹ Primary ovarian leiomyosarcoma (LMS) is an extremely rare subtype and accounts for about 0.1% of all ovarian cancers.² It mostly affects women between the ages of 45 and 60 years. These are highly aggressive neoplasm. The prognosis of women with primary ovarian LMS is very poor, with less than 20% of women alive at 5 years.² Here, we present a case of primary ovarian LMS with third-degree uterovaginal prolapse.

Case presentation

A P4L4 (parity 4, live 4) woman in her late 40s presented with chief complaints of something coming out per vaginum and lower abdominal pain for the last 5 years. There was no history of urinary complaints, altered bowel habits or any other comorbidities. Her menstrual cycles were regular. There is no family history of cancer in the first-degree relatives. For the above complaints, she consulted a local hospital where an ultrasound pelvis showed a large tubo-ovarian mass of 10×12 cm. So, she was referred to our tertiary care hospital for further management. On examination, she was Eastern Cooperative Oncology Group (ECOG) performance status 1; her vitals were stable. On abdominal examination, a 10×10 cm firm, non-tender pelvic mass with restricted mobility, occupying the whole hypogastrium and a part of the umbilical region felt. On local examination, third-degree uterovaginal prolapse was present. On per vaginal examination, the uterus was retroverted normal size. Same pelvic mass felt through fornices. Per rectal examination corroborated with per vaginal examination. Few firm to hard nodules were felt through the anterior rectal wall.

Investigations

Her routine haematological and biochemical investigations were within normal limits. Among tumour markers, her Cancer Antigen (CA) 125 was 55.6 U/mL, CA 19.9 was 22 058 U/mL and Carcino Embryonic Antigen (CEA) 0.66 ng/mL. Her upper Gastro-Intestinal (GI) endoscopy was normal, and her lower GI endoscopy showed external compression in the region of the recto-sigmoid junction. Her contrast-enhanced CT scan of the thorax, abdomen and pelvis showed a large solid cystic abdominopelvic mass of 7.4×10.3×9.7 cm posterior to the uterus, extending into bilateral adnexa (figure 1). Anteriorly mass was indenting postero-superior wall of the uterus and urinary bladder with indistinct planes, posteriorly abutting recto-sigmoid junction with loss of fat planes at places, superiorly mass was reaching pre sacral region with loss of fat planes with bowel loops. Bilateral ovaries were not seen separately from mass, suggestive of ovarian origin. The uterus was normal in size, shape and intensity. Uterovaginal descent with cystocele and rectocele was noted. Multiple omental deposits were also seen.

Figure 1.

Contrast-enhanced CT scan images of abdomen and pelvis showing ovarian mass. (A) Axial view, (B) coronal view, (C) sagittal view.

Treatment

Staging laparotomy with ascitic fluid cytology, total abdominal hysterectomy with bilateral salpingo-oophorectomy, suspension of vault with bilateral uterosacral ligaments, metastasectomy, excision of large tumour deposit over small intestine followed by resection and ileo-ileal anastomosis and omentectomy were done. Intraoperative images are shown in figure 2. Approximately 50 cc haemorrhagic ascites was present. The uterus was bulky, with the left ovary and tube grossly normal. Right ovarian mass 10×15 cm with tumour deposits over the surface along with multiple small subcentrimetric tumour deposits was present over the right fallopian tube. Small tumour deposit over urinary bladder peritoneum. Liver, under the surface of the diaphragm and spleen were grossly normal. 15×20 cm solid, multiloculated friable tumour mass was seen involving the ileum. Ileal resection (10 cm) and anastomosis were done using a linear bowel stapler and reinforced using 3–0 Primary Debulking Surgery (PDS). Multiple tumour deposits were present over small and large bowel, mesentery and parietal peritoneum (largest 4×3 cm). Multiple tumour deposits were present over the sigmoid colon and rectum. 5×3 cm tumour deposit was excised using ligasure, and seromuscular sutures were taken using 3–0 PDS. The appendix was grossly normal. Meticulous excision of various tumour deposits was performed.

Figure 2.

Intraoperative images. (A) Uterine prolapse with pelvic tumour, (B) pelvic mass, (C) pelvic mass orginating from right adnexa, (D) ovarian tumour infiltrating intestinal segment, (E) intestinal segment resection and anastomosis by staplers, (F) excised surgical specimen.

Intraoperative frozen section showed a malignant mesenchymal tumour. The total surgery duration was 4.5 hours. Intraoperative, two-unit packed Red Blood Cells (RBCs) were transfused. An intraperitoneal drain was put in view of extensive surgery along with intraoperative blood loss of about 1 L, generalised oozing at pelvic operative bed site and high chances of bowel anastomotic leak. Peritoneal Cancer Index score was 26. The Complete Cytoreduction score was 2. The postoperative period was uneventful. Histopathology showed stage III C ovarian LMS (figure 3) with immunohistochemistry report of Smooth Muscle Antigen (SMA) and Caldesmon diffuse cytoplasmic positive in tumour cells; CD34, PanCK, inhibin, CD10, P53, Desmin negative, Ki67 labelling index 15%–20%. The final diagnosis was a case of primary right ovarian LMS, International Federation of Obstetrics and Gynecology (FIGO) stage IIIC.

Figure 3.

Histopathology images. (A) H&E ×40 scanner view: normal ovarian parenchyma (right side green arrow corpus albicans) along with a tumour arranged in nodules and diffuse sheets. (B) H&E (×100): spindle cells tumour arranged in interlacing fascicles. (C) Higher magnification (×1000): spindle cells with marked cytological atypia, nuclear pleomorphism, cigar shaped nuclei with blunt ends and cytoplasmic vacuolation. These tumour cells are diffusely immunopositive for smooth muscle actin (SMA) (D), while negative for Desmin (E) and CD10 (F). (G) shows high Ki67 proliferative activity.

Differential diagnosis

The ovarian LMS is characterised histologically by spindle-shaped tumour cells organised in a whorled pattern, along with nuclear mitosis and necrotic areas. Immunohistochemical staining is found to be helpful for differentiating ovarian LMS from other spindle-cell tumours, such as fibrosarcoma or fibroma.^{3 4}

Outcome and follow-up

The patient was discharged on postoperative day 8. Her return to bowel activity and stitch line was normal. She was started on adjuvant chemotherapy on postoperative day 24 and was planned for six cycles of 3 weekly adriamycin and ifosfamide combination chemotherapy.

Discussion

This study presented a case of ovarian LMS with third-degree uterovaginal prolapse. Primary ovarian LMS accounts for less than 0.1% of ovarian sarcomas.² These smooth muscle tumours may arise from smooth muscle metaplasia with ovarian parenchyma or endometriosis, from the wall of ovarian vessels, or within teratoma.⁵ Primary ovarian LMS usually occurs in postmenopausal women; however, younger women may also be affected. In our cases, the patient is in the perimenopausal age group. Ovarian LMS does not have specific symptoms. Patients usually present with signs and symptoms related to pelvic mass, as in our case.

In literature, there are very few case series reported. Lerwill et al reported 54 smooth muscle tumours of the ovary, including 26 LMS. Seventy-one per cent developed recurrences at 19 months, and 62% died within 2 years.⁶ Another case series of 15 women with primary ovarian LMS is recently published by Cojocaru et al from the sarcoma unit at the Royal Marsden NHS Foundation Trust.² Twelve women presented with localised disease, and three women had metastatic disease at presentation. The median overall survival in advanced cases was 51 months.

Because of the rarity of primary ovarian LMS, there are no specific recommended guidelines in such cases. In most cases, complete cytoreduction is a cornerstone of treatment. Although other treatment modalities such as chemotherapy and radiotherapy have been described, there are no sufficient data to prove that such treatment will improve survival outcomes.⁷ The prognosis of these tumours is poor and mainly depends on the tumour size, stage and mitotic index.⁵

Patients perspective.

I came to this hospital in great agony after visiting many other hospitals. My surgery was done, and treatment was started promptly. My symptoms got resolved. I am very satisfied with the treatment provided in this hospital.

Learning points.

• Primary ovarian leiomyosarcoma is an extremely rare subtype of ovarian cancer.

• Immunohistochemistry, along with other histomorphological examination of tumour, is pivotal in confirming the diagnosis.

• The present case report provides essential information regarding the approach to diagnosis, the high metastatic rate of such tumours and treatment outcomes in the advanced stage. Even in advanced cases, surgical debulking with an attempt to R0 resection is found to provide good symptom relief, as in our case.

• Due to the dismal prognosis, women with ovarian leiomyosarcoma require early diagnosis and prompt intervention to improve symptoms and prolong survival.

• Because of the rarity of tumours and the lack of prospective studies, the standard management of such cases is yet to be established. However, radical surgery, followed by combined chemoradiotherapy and immunotherapy, has been considered in management in various case series.

Ethics statements

Patient consent for publication

Consent obtained directly from patient(s).